Dienst van SURF
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Heritable Connective Tissue Disorders (HCTD) show an overlap in the physical features that can evolve in childhood. It is unclear to what extent children with HCTD experience burden of disease. This study aims to quantify fatigue, pain, disability and general health with standardized validated questionnaires.METHODS: This observational, multicenter study included 107 children, aged 4-18 years, with Marfan syndrome (MFS), 58%; Loeys-Dietz syndrome (LDS), 7%; Ehlers-Danlos syndromes (EDS), 8%; and hypermobile Ehlers-Danlos syndrome (hEDS), 27%. The assessments included PROMIS Fatigue Parent-Proxy and Pediatric self-report, pain and general health Visual-Analogue-Scales (VAS) and a Childhood Health Assessment Questionnaire (CHAQ).RESULTS: Compared to normative data, the total HCTD-group showed significantly higher parent-rated fatigue T-scores (M = 53 (SD = 12), p = 0.004, d = 0.3), pain VAS scores (M = 2.8 (SD = 3.1), p < 0.001, d = 1.27), general health VAS scores (M = 2.5 (SD = 1.8), p < 0.001, d = 2.04) and CHAQ disability index scores (M = 0.9 (SD = 0.7), p < 0.001, d = 1.23). HCTD-subgroups showed similar results. The most adverse sequels were reported in children with hEDS, whereas the least were reported in those with MFS. Disability showed significant relationships with fatigue (p < 0.001, rs = 0.68), pain (p < 0.001, rs = 0.64) and general health (p < 0.001, rs = 0.59).CONCLUSIONS: Compared to normative data, children and adolescents with HCTD reported increased fatigue, pain, disability and decreased general health, with most differences translating into very large-sized effects. This new knowledge calls for systematic monitoring with standardized validated questionnaires, physical assessments and tailored interventions in clinical care.
The Sport Empowers Disabled Youth 2 (SEDY2) project encourages inclusion and equal opportunities in sport for youth with a disability by raising their sports and exercise participation in inclusive settings. The SEDY2 Inclusion Handbook is aimed at anybody involved in running or working in a sport club, such as a volunteer, a coach, or a club member. The goal of the handbook is to facilitate disability inclusion among mainstream sport providers by sharing SEDY2 project partners’ best practices and inclusive ideas.
In the Netherlands, client and family participation in care for people with intellectual disabilities has been in vogue for a long time, and increasingly receives attention (KPMG and Vilans 2017). However, the perspective and experiential knowledge of service users and relatives is often still insuBiciently used for the co-creation of care. The professional perspective is often still dominant. In addition, professionals mainly focus on clients and less on relatives, even though relatives often play an important role in the client’s (quality of) life (Wiersma 2017). The project ‘Inclusive Collaboration in Disability Care’[1] (ICDC) focusses on enhancing equal communication between people with intellectual disabilities, their relatives, and professional caregivers, and hence contributes to redressing power imbalances in longterm care. It investigates the question: “How can the triangle of client, relative and professional caregiver together co-create better care and support?”.